Presentation
Developmental delay.
Patient Data
A right parietal external ventricular drainage (EVD) tube is noted.
Absent septum pellucidum and corpus callosum agenesis are noted.
Bilateral asymmetrical dilatation of both occipital horns of lateral ventricles, the right side being larger, suggesting colpocephaly, along with prominent temporal horns.
Dilatation and upward extension of the third ventricle into the interhemispheric fissure are noted.
The fourth ventricle appears not dilated.
There is a large right parasagittal interhemispheric multilocular cystic lesion, with no obvious communication with lateral or third ventricles.
There is evidence of focal right parietal periventricular gray matter heterotopia.
No evidence of intra-cystic hyperintense T1W component or polymicrogyria.
Mega cisterna magna is noted. Unremarkable otherwise posterior fossa structures.
A right parietal external ventricular drainage (EVD) tube is noted.
Corpus callosum agenesis is noted.
Bilateral asymmetrical dilatation of both occipital horns of lateral ventricles, suggesting colpocephaly, along with prominent temporal horns.
The fourth ventricle appears not dilated.
A large right parasagittal interhemispheric multilocular cystic lesion has no obvious communication with lateral or third ventricles.
There is evidence of focal right parietal periventricular gray matter heterotopia.
No evidence of an intra-cystic hyperdense component.
Unremarkable posterior fossa structures apart from the mega cisterna magna.
Case Discussion
The Barkovich classification of interhemispheric cysts associated with agenesis of corpus callosum (ACC) includes type 1 cysts, which appear unilocular and communicate with lateral or third ventricles (diverticula), and type 2 cysts, which are often multiloculated and do not appear to communicate with the ventricular system 1.
Type 2 cysts are further divided on the basis of imaging appearance and co-existing morphology. Type 2c cysts are associated with subcortical heterotopia and thus the diagnosis in this instance 1.
Additionally, in the presence of asymmetric ventriculomegaly and dysgenesis of the corpus callosum along with the interhemispheric cyst, features are suggesting the triad of congenital cerebral anomalies: Asymmetric ventriculomegaly, interhemispheric cyst and dysgenesis of the corpus callosum (AVID).
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